BackgroundHypersensitivity reactions (HR) are common in mastocytosis. However, little is known about triggers and risk factors. The registry of the European Competence Network on Mastocytosis (ECNM) enables reliable studies in a larger cohort of mastocytosis patients. We assessed prevalence, triggers and risk factors of HR in adults with mastocytosis in the ECNM registry.MethodsData were collected in 27 ECNM centers. We analyzed potential triggers (Hymenoptera venoms, food, drug, inhalant and others) and risk factors at diagnosis and during follow‐up. The study group consisted of 2485 adults with mastocytosis, 1379 women (55.5%) and 1106 men (44.5%). Median age was 48.2 years (range 18–91 years).ResultsNine hundred and forty eight patients (38.1%) reported one or more HR`. Most common triggers were Hymenoptera venoms in cutaneous mastocytosis (CM) and indolent systemic mastocytosis (ISM), whereas in advanced SM (advSM), most common elicitors were drugs, including nonsteroidal anti‐inflammatory agents and penicillin. In multivariate analyses, tryptase level < 90 ng/mL, <15% infiltration by mast cells in bone marrow biopsy‐sections, and diagnosis of ISM were identified as independent risk factors for HR. For drug‐induced HR, prominent risk factors were advSM and high tryptase levels. New reactions were observed in 4.8% of all patients during 4 years follow‐up.ConclusionsHR are mainly triggered by Hymenoptera venoms in patients with CM and ISM and by drugs in patients with advSM. Tryptase levels <90 ng/mL, mast cell bone marrow infiltration <15%, and WHO category ISM are predictors of HR. New HR occur in 4.8% of all patients within 4 years.

中文翻译：

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各种形式的肥大细胞增多症中过敏反应的发生率：一项针对 ECNM 登记处收集的 2485 名成年肥大细胞增多症患者的初步研究

背景超敏反应（HR）在肥大细胞增多症中很常见。然而，人们对触发因素和风险因素知之甚少。欧洲肥大细胞增多症能力网络 (ECNM) 的注册能够在更大范围的肥大细胞增多症患者中进行可靠的研究。我们在 ECNM 登记处评估了肥大细胞增多症成人 HR 的患病率、触发因素和危险因素。方法数据收集于 27 个 ECNM 中心。我们分析了潜在的触发因素（膜翅目毒液、食物、药物、吸入剂等）以及诊断和随访期间的危险因素。研究小组由 2485 名患有肥大细胞增多症的成年人组成，其中 1379 名女性 (55.5%) 和 1106 名男性 (44.5%)。中位年龄为 48.2 岁（范围 18-91 岁）。结果 948 名患者 (38.1%) 报告了一项或多项 HR`。最常见的触发因素是膜翅目在皮肤肥大细胞增多症（CM）和惰性系统性肥大细胞增多症（ISM）中，最常见的诱导剂是毒液，而在晚期 SM（advSM）中，最常见的诱导剂是药物，包括非甾体抗炎药和青霉素。在多变量分析中，类胰蛋白酶水平 < 90 ng/mL、骨髓活检切片中肥大细胞浸润 <15% 以及 ISM 诊断被确定为 HR 的独立危险因素。对于药物引起的 HR，突出的危险因素是 advSM 和高类胰蛋白酶水平。 4 年随访期间，4.8% 的患者观察到新反应。结论 HR 主要由以下因素引发：膜翅目CM 和 ISM 患者使用毒液，advSM 患者使用药物。类胰蛋白酶水平 <90 ng/mL、肥大细胞骨髓浸润 <15% 和 WHO 类别 ISM 是 HR 的预测因子。 4.8% 的患者在 4 年内出现新的 HR。